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Cln1-mutations suppress Rab7-RILP interaction and impair autophagy contributing to neuropathology in a mouse model of INCL

Infantile neuronal ceroid lipofuscinosis (INCL) is a devastating neurodegenerative lysosomal storage disease (LSD) caused by inactivating mutations in the CLN1 gene. CLN1 encodes palmitoyl-protein thioesterase-1 (PPT1), a lysosomal enzyme that catalyzes the deacylation of S-palmitoylated proteins to...

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Bibliografske podrobnosti
izdano v:J Inherit Metab Dis
Main Authors: Sarkar, Chinmoy, Sadhukhan, Tamal, Bagh, Maria B., Appu, Abhilash P., Chandra, Goutam, Mondal, Avisek, Saha, Arjun, Mukherjee, Anil B.
Format: Artigo
Jezik:Inglês
Izdano: 2020
Teme:
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC8261861/
https://ncbi.nlm.nih.gov/pubmed/32279353
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/jimd.12242
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